tbuckley/cabot-search huggingface dataset:
P15 Progressive papulonodular eruption following COVID-19 infection: a rare diagnosis and review of the literature
Authors: Rhian Sheppeard, Stephanie Austin, Caroline Morgan, Margaret Ashton-Key
Journal: British Journal of Dermatology (2023)

Abstract We report a rare case of primary cutaneous CD8+ aggressive epidermotrophic cytotoxic T-cell lymphoma with striking clinical and histopathological features. A 51-year-old man, originally from Cameroon, presented to the department with a 6-week history of a progressive, itchy papulosquamous eruption that started on the lower abdomen shortly following infection with COVID-19. It became widespread over a short number of weeks, with blistering and ulceration in some areas. The patient reported fatigue with an intermittent low-grade pyrexia, but no systemic symptoms otherwise, and he was previously fit and well. Physical examination revealed an extensive, infiltrated papulonodular eruption with thick scale that was more marked in the skinfolds and involving the acral surfaces. Histology from three morphologically different sites showed marked epidermal hyperplasia and a prominent epidermotrophic infiltrate of medium-sized atypical lymphoid cells. There were frequent mitotic figures and evidence of apoptosis. Immunohistochemistry revealed that the atypical lymphoid cells in the epidermis were T cells expressing CD3, CD7 and CD8, together with cytotoxic markers and a high Ki67 proliferation fraction. These features were consistent with a diagnosis of CD8+ aggressive epidermotrophic cytotoxic T-cell lymphoma. Initial bloods and imaging were fairly unremarkable. Since the diagnosis, the patient has been treated with multiple types and courses of chemotherapy, with relapses in between. Most recently, the patient has been referred for total skin electron beam therapy with investigations for allogenic stem cell transplant ongoing. Owing to its rarity, primary cutaneous CD8+ aggressive epidermotrophic cytotoxic T-cell lymphoma is categorized only as a provisional entity in the most recent World Health Organization classification (Gilson D, Whittaker SJ, Child FJ et al. British Association of Dermatologists and U.K. Cutaneous Lymphoma Group Guidelines for the management of primary cutaneous lymphomas 2018. Br J Dermatol 2018; 180:496–526). Despite a variable and polymorphic presentation, the histology is distinctive in showing monomorphous, medium-sized atypical lymphocytes in the epidermis. We discuss the epidemiology of the disease and the paucity of therapeutic guidelines. In addition, our patient’s disease developed soon after COVID-19 infection; hence, we review the growing literature on the association between COVID-19 infection and vaccination and diagnosis, and relapse and remission of cutaneous T-cell lymphoma.
PA34 A case of disseminated herpes zoster in an immunocompetent child
Authors: Mareen Ann Jacob, Susan Kelly
Journal: British Journal of Dermatology (2023)

Abstract Herpes zoster (HZ) caused by the reactivation of varicella-zoster virus (VZV) affects mainly the adult population. The incidence is low in children (0.45 cases per 1000 individuals annually), especially in the immunocompetent. It is said to be disseminated when there are &amp;gt; 20 lesions outside the primary and adjacent affected dermatome. We present this case to highlight a rare complication of cutaneous dissemination in a previously healthy child with HZ, following in utero exposure to varicella at 28 weeks’ gestation. A 15-year-old boy presented with painful blisters in the T2 dermatome of 1 week’s duration, which later spread to trunk, extremities and face. This was associated with fever and neuralgia. There was no previous history of varicella and no recent contact history. He had no significant past medical history and was not on any medications. Antenatal history revealed that his mother had varicella at 28 weeks’ gestation and was treated with aciclovir. He was febrile on admission (39°C). There were coalesced vesiculopustules and crusted lesions with marked perilesional erythema in the T2 dermatome. In addition, disseminated vesicular lesions were seen on the trunk, extremities and face. Remaining systemic examination was unremarkable. C-reactive protein was elevated. Complete blood count and renal function tests were normal. Liver function tests, namely alanine transaminase and γ-­glutamyl transferase were elevated. Blood culture showed no growth. Varicella-zoster IgG was positive. Viral swab (polymerase chain reaction) from the skin lesions was positive for varicella-zoster DNA. Infectious serology for HIV, hepatitis B and hepatitis C was negative. Antinuclear antibody and antineutrophil cytoplasmic antibodies were negative. A clinical diagnosis of HZ with cutaneous dissemination was made, and he was treated with intravenous aciclovir. Intravenous antibiotics were added for suspected secondary bacterial infection. Disseminated zoster is less common in children and mainly occurs in patients with underlying immunodeficiency like HIV, immunosuppressive drug use or malignancy. In immunocompetent children, this can happen when primary infection (varicella) has occurred in utero or the first year of life due to the low response in specific varicella-zoster virus immunity. Complications like cutaneous dissemination due to viraemia are rare in healthy children, but can affect 2–10% of immunocompromised patients. It occurs 3–4 days after the onset of dermatomal lesions. It is important to look for other complications, especially in the immunocompromised, as viraemia can affect the brain, kidneys, lung and liver. Sequelae like postherpetic neuralgia is rare, but it is important to follow-up these children to look for its development.
BC03 Skincare in acne: are we letting our patients down?
Authors: Hamisha Salih, Saeeda Moradi, Pallavi Gupta
Journal: British Journal of Dermatology (2023)

Abstract Acne vulgaris is a chronic and recurrent inflammatory condition of the pilosebaceous unit. A combination of topical and systemic agents is often required to achieve symptom control, improve quality of life and reduce the risk of scarring. The role of skincare products in the management of acne has been well defined in the literature as a key adjunct to other therapies. We conducted an observational retrospective study to explore the patient experience of acne skincare advice provided by their dermatologist. Patients in the acne clinic, to which they would be referred for treatment with oral isotretinoin, were invited to participate. A survey incorporating multiple choice questions and an open comment box was distributed electronically. Twenty-nine per cent of patients acknowledged the importance of skincare products in managing their acne. Only 39% of respondents felt they had the opportunity to discuss skincare during their consultation. Over 86% of patients had been prescribed oral isotretinoin at least once; however, 60% had not received any skincare advice from their dermatologist. Only 31% of patients felt more confident with their skincare routine after their dermatology consultation than they did before. The internet and social media were the most popular sources of information for accessing skincare advice, with over 77% of patients identifying it as their main informant. Yet the majority of patients (88%) were likely to adhere to skincare advice if instructed by their dermatologist, clearly preferring this to social media. The most recurring theme from open comments was the desire to discuss skincare with their dermatologist and get signposted to a more reliable source of information for skincare. The data suggest that clinicians are failing to address or discuss skincare with patients, leaving them at the mercy of social media and at risk of exposure to scientifically unproven or incorrect advice. Integrating such advice when reviewing patients with acne would not only help with the management of their condition post-treatment with oral isotretinoin but also improve patient satisfaction and avoid misinformation. As a single-centre study, our results may not be representative of all patients, and the data collected were dependent on patient recall. Our results highlight the need for dermatologists to disseminate evidence-based skincare advice specific to acne-prone skin to their patients. Going forward, we aim to provide all our patients with acne with an information leaflet on skincare to complement their medical treatment.
DS06 Does the content of patient information leaflets reflect real-life practice? A comparison of four patient information leaflets in Mohs micrographic surgery
Authors: Sibel Demirel, Andrew Affleck
Journal: British Journal of Dermatology (2023)

Abstract In our experience, patients referred for Mohs micrographic surgery (MMS) often have unrealistic expectations, anticipating defects that are small in size and depth, and incorrectly describe the procedure as removal of skin cancer by ‘taking away thin slices’ or ‘skimming the skin layer by layer’. This observation prompted us to critically appraise four widely accessed online patient information leaflets (PILs) on MMS produced by the British Association of Dermatologists, the American Academy of Dermatology, Dermnetnz.org and the European Society of Micrographic Surgery. Our aim was to evaluate the wording used and propose evidence-based changes to help manage patients’ expectations. We focused on two core themes: (i) excision of tumour by MMS, including the number of stages, and (ii) cosmetic benefit of MMS over standard excision (SE). All of these PILs state the need for ‘stages’ to achieve margin clearance, with an initial margin of excision as low as 1 mm per stage mentioned in one PIL. The leaflets suggest cosmetic benefit and tissue preservation of MMS over SE, and one leaflet ranked this higher than other advantages offered by MMS. We feel that these points are overstated and better addressed with evidence-based facts. Studies have shown that 60% of cases of MMS are cleared in one stage; &amp;gt; 90% of tumours are cleared within two stages, and the number of patients needing three or more stages is only 6% (Macfarlane L, Waters A, Evans A et al. Seven years’ experience of Mohs micrographic surgery in a UK centre, and development of a UK minimum dataset and audit standards. Clin Exp Derm 2013; 38:262–9). In our experience, a surgical margin of 1 mm is not feasible in MMS. There is no direct evidence that MMS improves cosmesis compared with SE (Brown AC, Brindley L, Hunt WTN et al. A review of the evidence for Mohs micrographic surgery. Part 2: basal cell carcinoma. Clin Exp Dermatol 2022; 47:1794–804). Cosmetic and functional outcomes are usually only relevant in high-risk sites (e.g. the central face H zone) and in certain high-risk tumours where wide local excision would be the alternative treatment. In conclusion, we propose that PILs in MMS should avoid using misleading language like ‘thin’ and ‘small’ when describing the procedure. Patient information leaflets should clarify that the vast majority of tumours are cleared after two stages rather than requiring multiple stages. Tissue sparing should not be highlighted as the main advantage of MMS, and patients should not be pledged small wounds and better cosmetic outcomes.
BH06 (P100) Dermoscopy and clinicohistopathological profile of lichen planopilaris restricted to the face: a case series of six unusual cases
Authors: Rohit Kothari, Sunmeet Sandhu
Journal: British Journal of Dermatology (2023)

Abstract Lichen planopilaris (LPP) is an autoimmune cicatricial alopecia that usually affects the scalp. LPP confined to the face is rare and the information on its dermoscopy is limited. We present the dermoscopy and clinicohistopathological profile of six patients, all but one of whom were men aged 32–60 years. A 12-year-old girl was the youngest patient in our series, as well as in the literature to the best of our knowledge. All patients presented with varying degrees of hyperpigmented atrophic patches and scarring alopecia restricted to the face. Two had a linear morphology, which is usually the most common presentation of facial LPP. In our series, the chin was the most frequently affected site, followed by the cheek and upper lips; the forehead was the least frequently involved. None of the patients had lesions elsewhere on the body, including the scalp. The duration of the lesions ranged from 1 to 6 months. All cases showed a brown-to-violaceous background and/or mild blotchy erythema, significantly reduced hair follicles, cicatricial alopecia, and variable scaling and/or peripilar cast, brown dots, pigment pseudonetwork, pigment clumps and whitish scar-like areas on dermoscopy. Histopathology predominantly showed perifollicular inflammatory infiltrate, basal layer vacuolar degeneration and pigment incontinence, focal areas of perifollicular fibrosis, corresponding to the erythema and peripilar cast, brown dots and pigment clumps, and scar-like areas. The men all described regular shaving, almost daily. The girl and two men described having a regular mustard oil massage over the face, which might have irritated and damaged the facial skin. The possibility of continuous microtrauma due to shaving and mustard oil application acting as a trigger leading to development of facial LPP cannot be ruled out. All patients received hydroxychloroquine, potent topical corticosteroids, intralesional steroids and/or oral steroids. Patient 3 was lost to follow-up; the rest are under regular follow-up with a favourable—albeit suboptimal—response. This case series highlights a rare presentation of LPP restricted to the face, with only a few similar cases with dermoscopy reported in the literature. Its diagnosis requires a high degree of suspicion in any case presenting with scarring alopecia and atrophic patches over the face. The treatment is difficult and aims to halt disease progression and/or hair regrowth if detected and treated early, preventing significant morbidity and anxiety in the patient.
PA12 A case of pustular psoriasis in epidermolytic ichthyosis: coincidence or common pathway?
Authors: Fiona Lynch, Muriel Sadlier, Alan Irvine
Journal: British Journal of Dermatology (2023)

Abstract An 8-year-old boy with epidermolytic ichthyosis (EI) due to a heterozygous KRT1 mutation presented with a striking bullous skin eruption. Microbiological swabs were sent, and oral antibiotics were prescribed for suspected bullous impetigo. He deteriorated clinically, developing a fever and more extensive pustular rash reminiscent of generalized pustular psoriasis (GPP). Skin biopsies from two distinct cutaneous sites revealed histology strongly supportive of pustular psoriasis. Oral ciclosporin was commenced in addition to intensive topical emollient therapy. Following widespread desquamation, the pustular eruption fully resolved. Recurrence upon weaning of ciclosporin necessitated the introduction of ustekinumab. The rash remains controlled on this therapy. The EI is caused by heterozygous mutations in KRT1 or KRT10, and presents at birth with erythroderma and widespread skin denudation. The erythema and skin fragility abate in early childhood, giving way to prominent hyperkeratosis. Severe palmoplantar keratoderma is seen in those with KRT1 mutations. The GPP is a rare but life-threatening condition characterized by sterile pustules with or without systemic involvement. Interleukin (IL)-36-mediated inflammatory signalling plays a pathogenic role, as evidenced by loss of function mutations in IL36RN, which encodes an IL-36 receptor antagonist (Marrakchi S, Guigue P, Renshaw BR et al. Interleukin-36-receptor antagonist deficiency and generalized pustular psoriasis. N Engl J Med 2011; 365:620–8), and the efficacy of IL-36 receptor inhibitor spesolimab in a recent phase II trial. Both EI and GPP are rare, and the confluence of these entities in this case is striking. Four other cases in the literature describe a GPP-like eruption in patients with congenital ichthyoses. The coexistence of these conditions might be explained by their immunological profiles. Studies of the skin of patients with congenital ichthyoses have shown immune dysregulation with T helper 17/IL-23 skewing, and upregulation of associated inflammatory cytokines, including IL-36γ. The latter encodes for an IL-36 receptor agonist (Paller AS, Renert-Yuval Y, Suprun M et al. An IL-17-dominant immune profile is shared across the major orphan forms of ichthyosis. J Allergy Clin Immunol 2017; 139:152–65). Thus, increased IL-36 receptor agonist activity in congenital ichthyosis could theoretically lead to GPP in genetically predisposed individuals with already upregulated IL-36 receptor-mediated signalling.
DP01 Survey on the management of dysplastic naevus
Authors: Fazleenah Hussain, Arti Bakshi, Paul Devakar Yesudian, Stuart N Cohen
Journal: British Journal of Dermatology (2023)

Abstract Although dysplastic naevus (DN) is a frequent diagnosis, there is significant controversy on its definition and gradation and its relationship to melanoma risk. Therefore, there is no consensus over optimal management, and surveys from Australia, Canada and the USA have shown variation in clinical practice. Dysplastic naevus with involved histological margins has not been shown to progress to melanoma. To assess UK management of DN depending on the grade of atypia and reported excision margin, we circulated a questionnaire via email to dermatologists in Scotland, north Wales, Liverpool and Manchester. Seventy-two responded, of whom 46 (64%) were consultants. The cohort was generally highly experienced, with 10 (14%) responders with 1–5 years’ dermatology experience, 15 (21%) with 6–10 years, 27 (37%) with 11–20 years and 20 (28%) with &amp;gt; 20 years’ experience. Management of DN varied greatly. For mild DN excised with &amp;lt; 1-mm margin, 59 (82%) agreed that no further management was required. Opinions were divided when margins were involved in mild DN, with 14 (19%) agreeing that no further management was required, 42 (58%) choosing to re-excise and six (8%) to re-excising only any residual pigmentation. Most dermatologists reassured patients with moderately DN with margins &amp;lt; 1 mm (49, 68%); 18 (25%) preferred to re-excise. For moderate DN with involved margins, six respondents (8%) felt that no further management was necessary and 54 (75%) would re-excise. For severe DN with margins &amp;gt; 1 mm, 16 (22%) would still re-excise and six (8%) would agree management in the multidisciplinary team meeting (MDT). For severe DN with margins &amp;lt; 1 mm, 41 (57%) would re-excise and seven (10%) would agree management in the MDT. Lastly, for severe DN with involved margins, 62 (86%) would re-excise and seven (10%) would agree management in the MDT. This study has shown comparable results with those from Australia and the USA, whereby most dermatologists would re-excise moderate and severe DN with involved margins. Significant numbers indicated that they would re-excise in the case of close histological margins: this is unnecessary, and more standardized management may reduce expenditure. Limitations of the study include the usual potential sources of bias in a questionnaire survey; it should be noted that reported behaviour may differ from actual practice. However, this is the first UK study on this common clinical scenario. The move to report DN as low- or high-grade may help to reduce variation, but our survey suggests that some patients are currently undergoing unnecessary procedures.
DS07 (P22) Clinicopathologic characteristics of metastatic cutaneous squamous cell carcinoma: a cohort study
Authors: Nicholas Stefanovic, Alan Irvine, Niall Cosgrave, Lisa Killion, Patrick Ormond, Benvon Moran, Rupert Barry
Journal: British Journal of Dermatology (2023)

Abstract Cutaneous squamous cell carcinoma (cSCC) is a keratinocyte tumour with excellent prognosis following early-stage surgical treatment. Nevertheless, cSCC possesses an inherent potential for locoregional and distant metastasis in ∼4% of cases, contributing to disease-specific mortality. Recent guidance on cSCC management risk stratifies patients based on clinicopathological factors to identify those patients most at risk of metastasis. A retrospective cohort study identified 3455 cSCCs arising in 2522 patients, via review of histopathological diagnoses of cSCC at a tertiary hospital in Dublin, Ireland, between the years of 2009 and 2019. The aim of the study was to describe adequately a large cohort of patients with metastatic cSCC and investigate factors associated with metastasis. Metastatic disease was identified by histopathological and/or radiological confirmation in 116 patients, with complete data available in 105 cases. Twenty-two (21%) were female. Mean age at primary tumour diagnosis was 74.8 years, and mean time to first metastasis was 0.8 years. Twenty-six (25%) patients were immunosuppressed, the commonest reason for which was haematological malignancy. Median (SD) size of the primary tumour was 36 (21.65) mm, and median depth was 10 (7.43) mm. Eighty-six (82%) tumours were located on the head and neck. Seventeen (16%) of the tumours were recurrent, 58 (55%) were &amp;gt; 20 mm, 38 (36%) demonstrated extension beyond subcutaneous fat, 22 (21%) demonstrated lymphovascular invasion, 24 (23%) demonstrated perineural invasion of nerves &amp;gt; 0.1 mm in diameter, and 41 (39%) tumours were poorly differentiated. Twenty-six patients developed distant metastatic disease in addition to nodal metastasis. In most patients in our cohort, AJCC 8th Edition histopathological high-risk cSCC features were absent, apart from size &amp;gt; 20 mm. Conversely, most patients exhibited at least one clinical high-risk cSCC feature as per the British Association of Dermatologists guidelines for the management of cutaneous squamous cell carcinoma, including advanced age and tumour location (Keohane SG, Botting J, Budny PG et al. British Association of Dermatologists guidelines for the management of people with cutaneous squamous cell carcinoma 2020. Br J Dermatol 2021; 184:401–14). All patients were discussed at a specialist skin multidisciplinary team meeting, with significant variability noted in preoperative imaging, depending on the primary treating specialty. This cohort illustrates the importance of risk stratification of cSCC on clinicopathological features. This facilitates management decisions and ongoing patient surveillance with a view to preventing disease-specific morbidity and mortality.